Hemophilia is a hemorrhagic disorder with a sex-linked inherited pattern, characterized by an inability to amplify coagulation due to a deficiency in coagulation factor VIII (hemophilia A or classic) or factor IX (hemophilia B). Sequencing of the genes involved in hemophilia has provided a description and record of the main mutations, as well as a correlation with the various degrees of severity. Hemorrhagic manifestations are related to levels of circulating factor, mainly affecting the musculoskeletal system and specifically the large joints (knees, ankles, and elbows). This document is a review and consensus of the main genetic aspects of hemophilia, from the inheritance pattern to the concept of women carriers, physiopathology and classification of the disorder, the basic and confirmation studies when hemophilia is suspected, the various treatment regimens based on infusion of the deficient coagulation factor as well as innovative factor-free therapies and recommendations for the management of complications associated with treatment (development of inhibitors and/or transfusion-transmitted infections), or secondary to articular hemorrhagic events (hemophilic arthropathy). Finally, relevant reviews of clinical and treatment aspects of hemorrhagic pathology characterized by acquired deficiency of FVIII secondary to neutralized antibodies named acquired hemophilia.To determine the incidence of complications in elective surgeries and to grade them according to the Clavien-Dindo Classification System.
The cross-sectional study was conducted in the General Surgery Operation Theatre of Holy Family Hospital, Rawalpindi, Pakistan, from February to April 2018, and comprised patients undergoing elective surgeries. Age, gender, region of surgery, type and grade of complications, were recorded using Clavien-Dindo Classification proforma. Data was analysed using SPSS 23.
Of the total 212 patients, 36(16.9%) had some complication. There were significantly more complications in people aged 40 years or above compared to those &lt;40 years (p&lt;0.05). Of the total surgeries, 126(59.43%) were in the abdomino-pelvic region.
Peri-operative complications were found to be significantly related with age of the patient and the type of surgery.
Peri-operative complications were found to be significantly related with age of the patient and the type of surgery.Breast cancer remains the leading cause of cancer related death in females worldwide. Metastatsis from breast primary are usually seen in lungs, bones and liver. Uncommon sites include adrenals, thyroid, spleen, pancreas and urinary bladder. Retroperitoneal metastasis are considered most unsual among breast cancer. We present the case of a treated breast cancer patient with a large reteroperitoneal lesion suggesting other aetiologies on imaging but proved to be metastasis on histopathology.Optimal management of craniopharyngioma is challenging and requires multidisciplinary efforts for a successful outcome. The debate over radical versus a conservative resection followed by radiotherapy, continues. Current literature reports no difference in disease freedom and overall survival between the two surgical viewpoints, and favours conservative resection and radiotherapy, leading to lower morbidity and superior functional outcomes.This communication describes a contemporary model of diabetes monitoring and therapy assessment, the Time-in-range'(TIR), as the Glycaemic compass. The term 'compass' is apt, as it reflects the dynamic nature of TIR concept. TIR can be used not only as a target, but also as a tool to achieve this target, to explain the importance of good control, and to facilitate changes in therapeutic approach. The theranostic nature of TIR allows it to be termed as a compass for glycaemic control.This review on endocrine fatigue syndrome (EFS) describes the various clinical presentations of fatigue in endocrinopathy. It enumerates the various endocrine and non-endocrine causes of fatigue in endocrinopathy, and shares pragmatic clinical tips to help in differential diagnosis.People who inject drugs (PWIDs) reuse injection equipment and other paraphernalia, which is a major source of transmission of human immunodeficiency (HIV) and hepatitis C virus (HCV) infections. PWIDs who use infected injections develop many surgical complications which are life- threatening. The most common complication is infected pseudoaneurysm. To assess complications requiring surgery in drug abusers, a study was conducted in the East Surgical Ward of Mayo Hospital Lahore, Pakistan from Jan 2017 to Jan 2019. A total of 48 PWIDs presented to the surgical department, out of which 39 (81%) were HIV positive, and 31 (64%) were HCV positive. Of the 52% who had pseudoaneurysm, 92% had femoral and popliteal aneurysm and 8% had brachial aneurysm. After ligation and excision of pseudoaneurysm only 8% required major amputation. Mortality rate was 0%. Ligation and excision is a safe option in pseudoaneurysm. Most common site for pseudoaneurysm was left femoral artery.First described in 1934, eccrine spiradenoma (ES) is a rare, benign adnexal tumour arising from eccrine sweat glands. https://www.selleckchem.com/products/namodenoson-cf-102.html It commonly presents as a slow-growing nodule on the upper trunk, and head and neck region, mostly in the age bracket of 15-35 years, with no gender preference. While no established guidelines exist for optimal management of malignant ES, some therapies have been studied. The diagnosis of this entity is extremely important as it can harbour a malignant component with disastrous outcomes which may be missed due to its strong resemblance to benign lesions, such as a papilloma. Here, we present the case of a 35-year-old lady who presented with a papilloma-like growth on the upper medial aspect of the thigh which was diagnosed as eccrine spiradenoma upon excision.We report two treatment-naïve cases, a 26-year-old female patient and a 59-year-old male patient who were followed up for chronic hepatitis B (CHB) at the department of Infectious Diseases and Clinical microbiology. A partial response subsequent to 12 months of Tenofovir Disoproksil (TDF) monotherapy presumably due to an antiviral-drug resistance was noted. A sustained viral response with TDF (245 mg) or Tenofovir Alafenamide (TAF, 25 mg) + Entecavir (ETV, 1 mg) combination therapy was observed after failure with TDF monotherapy. A combination therapy with TDF (245 mg) or TAF (25 mg) +ETV (1 mg) is efficacious in naïve patients with a partial response to TDF monotherapy.